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Prevalence of suspected collagen disorders in the adult bleeding disorder clinic
Abstract number: PP-WE-695
Jackson1 S.C., Card1 R.T., Brown1 M., Spitzer1 C., Poon1 M.
11Department of Medicine, Division of Hematology, University of Calgary, Calgary, Canada
How-to-cite Jackson SC, Card RT, Brown M, Spitzer C, Poon M. Prevalence of suspected collagen disorders in the adult bleeding disorder clinic. Journal of Thrombosis and Haemostasis 2009; Volume 7, Supplement 2: Abstract PP-WE-695
Background: Disorders of collagen may be associated with a mild bleeding tendency because of the interaction of collagen with von Willebrand factor (VWF) and platelets during primary hemostasis. Collagen disorders are often associated with joint hypermobility and may be unrecognized contributors to existing disorders of primary hemostasis resulting in more bleeding than predicted based on laboratory abnormalities alone.
Objective: To determine the prevalence of suspected collagen disorders in a cohort of adult subjects ≥ 17 years of age attending the Southern Alberta Adult Bleeding Disorders Clinic.
Methods: Consecutive subjects were enrolled between June 2008 to January 2009 who had a provisional diagnosis of von Willebrand disease, low VWF levels, mild platelet function disorders or undefined bleeding disorders. Condensed Vincenza Bleeding Score, Beighton exam for hypermobility and Brighton criteria were performed on each subject. Retrospective data on bleeding time, closure times, VWF:Ag, VWF:RCo, FVIII:C and platelet function were collected.
Results: Out of 32 subjects (30 female, 2 male) who completed the study, 11 (34%) met the major Brighton criteria for Benign Joint Hypermobility Syndrome (BJHS). Sixty four percent (7/11) had a personal or family history of Ehlers-Danlos Syndrome (n = 5), BJHS (n = 1), or Osteogenesis Imperfecta (n = 1). All of these individuals were registered to the clinic as provisional VWD (5), mild platelet function disorder (1) or undefined bleeding disorder (1). When compared to the 21 subjects who did not meet major criteria for BJHS, there was a trend towards higher VWF:Ag, VWF:RCo and FVIII in the suspected collagen disorder group but no difference between groups for age, bleeding times, or bleeding scores.
Conclusion: Suspected disorders of collagen may be common in the bleeding disorders clinic and further study is required to determine if this contributes to the bleeding phenotype.
Disclosure of interest: none declared.
To cite this abstract use the following format:
Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number
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